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Hyperimmunoglobin E syndrome: a sign of TH1/TH2 imbalance?

We report on a patient with hyperimmunoglobulin E syndrome, who developed pruritic vesiculopapules from the age of six months and also had recurrent episodes of skin abscesses and oral thrush. Serum IgE was extremely elevated at 59,514 IU/ml and specific IgE antibody to Staphylococcus aureus was positive. Histological examination from a vesiculopapule on the face revealed that eosinophil-rich infiltration involved hair follicles, similar to eosinophilic pustular folliculitis. We also examined cytokine profiles of circulating CD4+ T cells by intracellular cytokine staining and flow cytometry. The ratio of cells positive for interferon-gamma was significantly reduced compared with a control. Several reports have shown decreased interferon-gamma production by peripheral blood mononuclear cells of patients with hyperimmunoglobulin E syndrome. We think that this cytokine profile and the histological findings of our patient support the hypothesis that TH1/TH2 imbalance is involved in hyperimmunoglobulin E syndrome.

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